Factor X and its deficiency states

Perry, DJ

doi:10.1046/j.1365-2516.1997.00106.x

Cited by 44 publications

(50 citation statements)

References 69 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A secondary rise to 173% occurs at 144hours postpartum, the level again approaching the non-pregnant levels at 6weeks after delivery 1 Inherited Factor X (FX) deficiency is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:500000. 2 Factor X deficiency is more common in parts of the world where there is increased incidence of consanguinity. 3 The prevalence of heterozygous FX deficiency (carrier state) may be as high as 1:500.…”

Section: Discussionmentioning

confidence: 99%

Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

Venkatesh¹,

Ross²,

Vani³

2017

IPCB

View full text Add to dashboard Cite

Abbreviations: FX, factor X; FX,C, factor X coagulant; FFP, fresh frozen plasma; PCC, prothrombin complex concentrate Case reportA 23year old lady with one previous abortion, presented to the antenatal outpatient department of St. John s Hospital, for the first time at 20weeks of gestation. She gave a history of prolonged bleeding following trivial trauma since childhood which stopped on compression. She also had hemarthrosis and spontaneous gum bleeds since childhood. At puberty, she gave history of menorrhagia which responded to oral Tranexamic acid. At 17years of age, patient had a severe episode of menorrhagia leading to hemorrhagic shock. She received multiple blood products and eventually required bilateral uterine artery ligation and a few haemostatic sutures on the uterus (exact details of which are not known and have not been documented). Soon after marriage, at 21years of age, she was diagnosed to have blighted ovum and presented later with incomplete abortion, for which she received Misoprostol. She had heavy bleeding leading to severe anemia and a provisional diagnosis of disseminated intravascular coagulation was made. Her investigations at that time were Prothrombin time 102s, Activated thromboplastin time 65.5 s, INR 8.5, Factor X levels were <1% with normal platelet count and morphology. Thus, a diagnosis of severe factor X deficiency was made. During the present pregnancy, she was referred to the antenatal outpatient department. She had developed gestational diabetes mellitus which was controlled with diet. The antenatal period was uneventful. As there was no significant bleeding in this pregnancy, the coagulation parameters were not repeated until term. Ultrasound done at 37weeks confirmed a flexed breech presentation. At 37weeks of gestation, patient received FFPs at 1 bag/10kg body weight to assess her laboratory haemostatic parameters and her investigations were as shown in Table 1. Elective cesarean delivery was planned at 38weeks of gestation and external cephalic version was not considered due to the previous scar on the uterus (i.e. haemostatic sutures on the uterus following an episode of menorrhagia at 17years of age). Patient underwent emergency cesarean delivery at 37weeks of gestation, as a spontaneous retro placental bleed of about 50ml was seen on ultrasound. Patient again received the same dose of FFP and 2g of intravenous Tranexamic acid preoperatively. A healthy baby boy of weight 2.5kg was delivered as flexed breech. A previous scar was noted on the uterus. Both ovaries were normal. Intraoperatively, a peritoneal drain was left in situ. Patient developed hematuria which cleared after 12hours. Postoperatively, patient received 3units of FFP and 1g intravenous Tranexamic acid 8 hourly for 48hours and then followed by 2units of FFP and 1g intravenous Tranexamic acid 12hourly till the seventh postoperative day. Sutures were removed on the seventh postoperative day. Postoperative period was uneventful. Patient has come for follow-up and has had no signs of postpartum hemorrhage til...

show abstract

Section: Discussionmentioning

confidence: 99%

Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

Venkatesh¹,

Ross²,

Vani³

2017

IPCB

View full text Add to dashboard Cite

show abstract

“…8 Functional activity of Factor X (FX: C) is quantified by performing prothrombin time e based assay, using rabbit thromboplastin and Factor X deficient plasma. Accordingly, patients are classified into three groups: severe (FX: C, <1%), moderate (FX: C, 1%e4%) & mild (FX: C, 6%e 10%).…”

Section: Discussionmentioning

confidence: 99%

Inherited Factor X (Stuart–Prower Factor) deficiency and its management

Chatterjee

Philip

Nair

et al. 2015

Medical Journal Armed Forces India

View full text Add to dashboard Cite

“…FX deficiency is one of the rarest coagulation disorder estimated at about 1 in every 1 or 2 million people [3,4,6]. It is also worth noting that none of the 21 homozygous or double heterozygous patients studied in Padua (including 12 cases of FX Friuli disorders) were associated with clotting defects.…”

Section: Discussionmentioning

confidence: 99%

“…In 1975 we had gathered 25 cases [2]. Since then severe additional cases [3][4][5][6] have been reported. Recent papers and reviews have gathered and discussed approximately an additional 50 cases.…”

Section: Introductionmentioning

confidence: 99%

Congenital FX deficiency combined with other clotting defects or with other abnormalities: a critical evaluation of the literature

et al. 2007

View full text Add to dashboard Cite

The presence of more than one congenital clotting defect in a given patient is a rare event but not an exceptional one. Combined defects of factor X (FX) are very rare because congenital isolated FX deficiency is by itself very rare. A perusal of personal files and of the literature has yielded 12 families with FX deficiency in which an association with another clotting factor deficiency was found. The associated defects were factor VII (FVII) or factor VIII (FVIII) or factor XII (FXII) deficiency. By far the most frequently associated was with FVII. Two forms of this association were found. In the first form there is casual association of both FVII and FX deficiency in the proband with independent recessive segregation of the two defects in other family members. The second form is because of abnormalities in chromosome 13 (deletions, translocations and so on) involving both FX and FVII genes. These genes are known to be very close and located on the long arm of chromosome 13 at about 13q34. In this form the hereditary pattern is autosomal dominant. Isolated FX deficiency and, more frequently, combined FX + FVII deficiency appear also associated with coagulation-unrelated abnormalities (carotid body tumours, mitral valve prolapse, atrial septal defect, ventricular septal defect, thrombocytopenia absent radius (TAR) syndrome, mental retardation, microcephaly and cleft palate). Diagnosis of a combined clotting defect could be difficult on the basis of global tests. For example, both isolated FX deficiency and combined FX + FVII deficiency yield a prolongation of basal PTT and PT. Only specific assays could allow one to reach the correct diagnosis. In cases of casual association with other defects, it is also important to study family members, as the two defects should segregate independently.

show abstract

Factor X and its deficiency states

Cited by 44 publications

References 69 publications

Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

Severe Factor X Deficiency in Pregnancy-An Obstetric Challenge

Inherited Factor X (Stuart–Prower Factor) deficiency and its management

Congenital FX deficiency combined with other clotting defects or with other abnormalities: a critical evaluation of the literature

Contact Info

Product

Resources

About